What is the life expectancy after diagnosis of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD)?

Life Expectancy in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD)

MOGAD is not associated with reduced life expectancy compared to the general population, but approximately 40-50% of patients will experience disease relapses that can lead to disability if not properly treated.

Disease Overview and Prognosis

MOGAD is a recently identified autoimmune demyelinating disorder of the central nervous system characterized by antibodies targeting myelin oligodendrocyte glycoprotein. Unlike some other demyelinating conditions, MOGAD does not appear to significantly impact mortality but can affect quality of life through relapses and potential disability.

Key Prognostic Factors

• Relapse Risk:

  • Approximately 40-56% of patients experience at least one relapse 1, 2
  • Highest risk of relapse occurs early in the disease course 1
  • Median time to first relapse is 3.2 months, but can range from 1 to 86 months 1

• Long-term Disability:

  • At long-term follow-up (median 77.8 months), only 17.2% of patients had moderate disability (EDSS ≥3) 1
  • Severe disability (EDSS ≥6) was observed in only 4.7% of patients 1
  • Most patients have good functional recovery between attacks

Factors Affecting Prognosis

Positive Prognostic Factors

1. Early immunotherapy (within 7 days from onset) - associated with 6.7-fold reduced odds of developing a relapsing disease course 3

2. Longer corticosteroid treatment (≥5 weeks) - associated with 6.7-fold reduced odds of relapse 3

3. Maintenance therapy initiation after first attack - reduces relapse risk significantly (OR = 0.26) 1

   • 2-year relapse risk: 11% with early maintenance vs. 41% without
   • 8-year relapse risk: 20% with early maintenance vs. 56% without

4. Disappearance of MOG-IgG antibodies after initial attack may indicate better prognosis with lower relapse risk 4

Negative Prognostic Factors

1. Higher disease severity at onset - each 1-point increment in worst EDSS at onset associated with 6.7-fold increased odds of final disability 3

2. Relapsing disease course - associated with higher proportion of disability (37.5% vs. 12.8% in monophasic disease) 3

3. Persistent MOG-IgG antibodies - patients with relapsing disease typically maintain detectable antibodies 4

Clinical Presentation and Monitoring

MOGAD most commonly presents as:

• Optic neuritis (53-63% of cases) 5, 1
• Transverse myelitis (4-20% of cases) 5, 1
• Brainstem encephalitis/rhombencephalitis (17%) 5
• ADEM-like presentations (particularly in children) 3

Monitoring Recommendations

1. Antibody testing: Consider re-testing MOG-IgG 6-12 months after initial attack to assess persistence, which may have prognostic implications 4

2. Regular clinical follow-up: Particularly important during the first year when relapse risk is highest 2

3. MRI monitoring: To detect subclinical disease activity

4. Red flags that should prompt re-evaluation of diagnosis 4:

   • Chronic progressive disease course (very rare in MOGAD)
   • Lesion patterns typical of MS (periventricular ovoid lesions, Dawson's fingers)
   • Combined central and peripheral demyelination
   • Positive MOG-IgM/IgA with negative MOG-IgG

Treatment Impact on Prognosis

The most recent evidence strongly suggests that early initiation of maintenance therapy significantly improves long-term outcomes:

• Maintenance therapy options include rituximab, IVIG, mycophenolate mofetil, and ocrelizumab 2
• Relapse risk reduction: 8-year relapse risk of 20% with early maintenance therapy vs. 56% without 1

Conclusion

MOGAD does not appear to significantly reduce life expectancy, but quality of life can be impacted by relapses and resulting disability. Early and appropriate treatment, particularly initiation of maintenance therapy after the first attack, significantly improves long-term outcomes by reducing relapse risk and subsequent disability.